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Pirfenidone for IPF: pro/con debate; the ‘con’ viewpoint
  1. Ganesh Raghu1,
  2. David R Thickett2
  1. 1 Division of Pulmonary & Critical Care Medicine, University of Washington (UW), Scleroderma Clinic, UW Medical Center, Seattle, Washington, USA
  2. 2 Centre for Translational Inflammation and Fibrosis Research, School of Clinical and Experimental Medicine, University of Birmingham, UK
  1. Correspondence to Dr David Thickett, Centre for Translational Inflammation and Fibrosis Research, School of Clinical and Experimental Medicine, University of Birmingham, UK; d.thickett{at}

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Improving outcomes for patients with idiopathic pulmonary fibrosis (IPF) remains a major unmet healthcare need—in this, we entirely agree with the ‘pro’ debate article by Gisli Jenkins.1 In this regard, the efforts of drug companies in developing novel drugs and collaboration with expert clinical investigators in the field of IPF in wanting to demonstrate the therapeutic efficacy for patients with IPF are commendable.

Unfortunately, several clinical trials of novel therapy for IPF have yielded negative results. In 1999, the therapeutic potential of pirfenidone as the first antifibrotic pharmacologic agent with promises/hopes that it would improve outcomes for IPF patients was demonstrated in a phase II study.2 Some 13 years later, initial enthusiasm is being questioned—hence, the rationale for this pro/con debate. In this article, it is our aim to provide a balance to Dr Gisli Jenkins's pro article,1 and we hope to provide the readers of this debate a balanced view of the topic. Undoubtedly, the reader will realise that the truth probably exists between the two ends of this polarised academic debate. We believe that the clinician must assess the benefits and risks of pirfenidone treatment by addressing the following specific issues.

IPF has complex pathophysiology

Current evidence suggests that in a genetically predisposed person, IPF is manifested as a result of an aberrant response to an unidentified alveolar epithelial injury. Theories speculate that IPF results from abnormal wound-healing response which results in a persistently abnormal epithelial repair that promotes fibroblast proliferation generating a reticulum of activated fibroblasts and collagen which progressively restructures the lung architecture.3 ,4 In addition to myofibroblast foci formation and epithelial cell injury, there is variable evidence of inflammation as evidenced by increased macrophage and neutrophil counts,5 ,6 intra-alveolar coagulapathy,7 and the formation of new blood vessels in the IPF lung.8 …

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